1389 Unusual Presentation of an Intraductal Papillary Neoplasm of the Bile Duct (IPNB) Masquerading as Mirizzi Syndrome Correctly Diagnosed With SpyGlass Cholangioscopy

Abstract

INTRODUCTION: Intraductal papillary neoplasm of the bile duct (IPNB) is relatively rare in the Western world with higher incidence reported in Far Eastern countries because endemic hepatolithiasis and clonorchiasis. We describe an unusual presentation of the tumor masquerading as Mirizzi syndrome. CASE DESCRIPTION/METHODS: A 64-year-old lady with a history of cholelithiasis presented to the hospital with acute onset of severe epigastric and right upper quadrant pain with associated symptoms of nausea and vomiting. She was afebrile and was noted to have epigastric and right upper quadrant tenderness. She had abnormal liver enzymes with AST of 958, ALT of 994, alkaline phosphatase of 170 and bilirubin of 1.5 that increased to 3.9. An ultrasound revealed cholelithiasis and a positive sonographic Murphy's sign. There was intrahepatic and extrahepatic biliary ductal dilation. Debris was noted within a 9 mm diameter bile duct. A HIDA scan revealed non-visualization of the gallbladder at 4 hours compatible with acute cholecystitis/cystic duct obstruction. MRI with gadolineum and MRCP revealed a 17 mm irregular shaped filling defect within the cystic duct with extension into the junction of the common hepatic and common bile duct most consistent with a large stone, Mirizzi syndrome to be considered. It also revealed cholelithiasis and gallbladder wall edema consistent with acute cholecystitis. A ERCP was performed that revealed a filling defect causing obstruction at the CHD/CBD/cystic duct junction thought to be caused by a large stone impacted within the cystic duct. A sphincterotomy was performed and multiple balloon sweeps were done without retrieval of a stone. A 10 F/9 cm size plastic stent was placed with complete resolution of her symptoms. She was scheduled for A SpyGlass cholangioscopy for further evaluation and possible electrohydraulic lithotripsy/stone extraction. SpyGlass cholangioscopy interestingly revealed a tumor occluding the cystic duct and extending into the CHD and CBD causing luminal narrowing. The tumor had frond-like papillary projections characteristic of an IPNB. Biopsies obtained with SpyBite biopsy forceps confirmed IPNB. The patient underwent tumor resection along with cholecystectomy and Roux-en-Y hepaticojejunostomy with excellent recovery. DISCUSSION: Peroral cholangioscopy with biopsies provided a definite diagnosis in this patient with an acute Mirizzi syndrome like presentation of IPNB leading to appropriate surgical management.